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While the etiology of the disease is unknown, recent genetic studies have demonstrated that an X\u2010linked form of familial cardiac valvular dystrophy can be attributed to mutations in the Filamin\u2010A gene. Since these inheritable mutations are present from conception, we hypothesize that filamin\u2010A mutations present at the time of valve morphogenesis lead to dysfunction that progresses postnatally to clinically relevant disease. Therefore, by carefully evaluating genetic factors (such as filamin\u2010A) that play a substantial role in MVP, we can elucidate relevant developmental pathways that contribute to its pathogenesis. In order to understand how developmental expression of a mutant protein can lead to valve disease, the spatio\u2010temporal distribution of filamin\u2010A during cardiac morphogenesis must first be characterized. Although previously thought of as a ubiquitously expressed gene, we demonstrate that filamin\u2010A is robustly expressed in non\u2010myocyte cells throughout cardiac morphogenesis including epicardial and endocardial cells, and mesenchymal cells derived by EMT from these two epithelia, as well as mesenchyme of neural crest origin. In postnatal hearts, expression of filamin\u2010A is significantly decreased in the atrioventricular and outflow tract valve leaflets and their suspensory apparatus. Characterization of the temporal and spatial expression pattern of filamin\u2010A during cardiac morphogenesis is a crucial first step in our understanding of how mutations in filamin\u2010A result in clinically relevant valve disease. Developmental Dynamics 239:2118\u20132127, 2010 \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/dvdy.22346","type":"journal-article","created":{"date-parts":[[2010,6,24]],"date-time":"2010-06-24T03:23:05Z","timestamp":1277349785000},"page":"2118-2127","source":"Crossref","is-referenced-by-count":50,"title":["Expression of the familial cardiac valvular dystrophy gene, filamin\u2010A, during heart 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